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Obviando cirurgia major : diagnóstico diferencial de IgG4-SC
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Resumo(s)
Colangite eslerosante associada às imunoglobulinas G4 (IgG4-SC) é uma entidade clínica rara, recentemente descrita na literatura, de provável etiologia autoimune. É reconhecida como manifestação biliar da doença associada às imunoglobulinas G4 (IgG4-RD) e tem uma boa resposta à corticoterapia.
A sua etiologia, história natural da doença, incidência e prevalência, fatores de risco e fatores protetores associados, biomarcadores diagnósticos e efeitos a longo prazo ainda permanecem pouco esclarecidos.
Níveis séricos de IgG4, achados imagiológicos característicos, coexistência de doença associadas às IgG4 noutros órgãos e achados histopatológicos característicos permitem um diagnóstico atempado da IgG4-SC.
O diagnóstico diferencial desta entidade patológica com a neoplasia maligna hepato-bilio-pancreática e com a colangite esclerosante primária é complexo, sendo ainda mais difícil perante a ausência de outras manifestações multissitémicas da IgG4-RD, nomeadamente pancreatite autoimune (AIP).
Este trabalho de revisão da literatura e dos estudos de grupos de investigação internacionais tem como objetivo alertar os profissionais de saúde para a existência desta entidade patológica que deve ser integrada no diagnóstico diferencial com as doenças de abordagem terapêutica mais invasiva, uma vez que a sua confirmação permite obviar uma cirurgia major desnecessária.
Immunoglobulin-G4 related slerosant cholangitis (IgG4-SC) is a rare clinical entity, recently described in the literature, with probable autoimmune etiology. It is recognised as a biliary manifestation of the immunoglobulin-G4 related disease (IgG4-RD) and has a good response to corticosteroids. Its aetiology, pathogenesis, incidence and prevalence, risk factors and associated protective factors, diagnostic biomarkers and long-term effects remain unclear. Serum levels of IgG4, characteristic imaging findings, coexistence of IgG4-associated disease in other organs and characteristic histopathological findings are useful for diagnosis of IgG4-SC. The differential diagnosis of this pathological entity with the malignant hepato-biliary-pancreatic malignancy and primary sclerosing cholangitis (PSC) is complex and is even more difficult in the absence of other multisitemic manifestations of IgG4-RD, such as autoimmune pancreatitis (AIP). This review of the literature and international research groups studies aims to alert physicians and surgeons to the existence of this pathological entity, which must be integrated in the differential diagnosis with the patological entities, whose therapeutic approach is more invasive, since its confirmation allows to obviate unnecessary major surgery.
Immunoglobulin-G4 related slerosant cholangitis (IgG4-SC) is a rare clinical entity, recently described in the literature, with probable autoimmune etiology. It is recognised as a biliary manifestation of the immunoglobulin-G4 related disease (IgG4-RD) and has a good response to corticosteroids. Its aetiology, pathogenesis, incidence and prevalence, risk factors and associated protective factors, diagnostic biomarkers and long-term effects remain unclear. Serum levels of IgG4, characteristic imaging findings, coexistence of IgG4-associated disease in other organs and characteristic histopathological findings are useful for diagnosis of IgG4-SC. The differential diagnosis of this pathological entity with the malignant hepato-biliary-pancreatic malignancy and primary sclerosing cholangitis (PSC) is complex and is even more difficult in the absence of other multisitemic manifestations of IgG4-RD, such as autoimmune pancreatitis (AIP). This review of the literature and international research groups studies aims to alert physicians and surgeons to the existence of this pathological entity, which must be integrated in the differential diagnosis with the patological entities, whose therapeutic approach is more invasive, since its confirmation allows to obviate unnecessary major surgery.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2019
Palavras-chave
Colangite esclerosante associada às IgG4 Imunoglobulinas G4 Doença associada às IgG4 Colangite esclerosante primária Colangiocarcinoma